Patient with CRF of unknown etiology who was on HD for 2 years, without complications, except for peripheral polyneuropathy.
His wife's kidney was injured at the age of 61 in October 1994.
Treatment consisted of cyclosporine, azathioprine and prednisone.
Diltiazem and ketoconazole were used together to reduce the daily dose of cyclosporine for economic reasons.
One year after KTx, creatinine was 1.22 mg/dl and creatinine clearance was 92.2 ml/min/1.73 m2.
During the first 5 years of KTx, she suffered paroxysmal atrial fibrillation (which resolved with amiodarone, which was prescribed on admission), episodes of allergic rhinitis, bronchial asthma, gastroesophageal reflux.
At the fifth year of KTx, creatinine was 1.28 mg/dl and there was no proteinuria.
Between the 5th and 10th year of the KTx, the function of the graft remained stable, appearance of respiratory fistulae and genital condyloma acuminata, suffered a fracture due to bronchial obstruction of a vertebral body.
At the tenth year creatinine was 1.4 mg/dl, creatinine clearance 65 ml/min/1.73 m2, proteinuria 300 mg/24 h.
Between the 10th and 15th year of the KTx, there were no changes in the graft, presented several episodes of carotid hernia, occluded an umbilical hernia, diffuse hetero-perfectiasis and symptomatic iliac-femoral bypass.
Creatinine at 15° of KTx was 1.2 mg/dl, creatinine clearance 66.8 ml/min/1.73 m2 and proteinuria 130 mg/24 h.
One year later a severe calcified aortic stenosis was diagnosed with a systolic gradient of 68 mmHg, with surgical indication.
The patient decided to undergo surgery only in case of vital risk.
In the following 3 years, renal function remained stable and aortic stenosis did not progress in periodic echocardiographic evaluations.
She also had a self-limiting trigeminal neuralgia and suffered a crisis with short evolution, in which chest X-rays showed no pulmonary alterations.
At 19 years of KTx, a chest X-ray taken during a cardiac evaluation showed interstitial infiltrates that led to a chest scan.
This showed diffuse calcifications in both lungs that were clinically associated with occasional dry cough and poor morning mucosal expectoration (the patient smoked until he was admitted to HD when he discontinued the habit for 12 weeks).
Since the patient had not suffered from lung diseases that could cause dystrophic calcifications, it was interpreted that these corresponded to MPS.
A study of pulmonary function and biochemical determinations related to calcium metabolism (which were not previously available) were requested.
However, the patient died suddenly from an acute myocardial infarction in an emergency department, weeks after the diagnosis of MPS and failed to perform them.
Until his last medical control, he used prednisone, cyclosporine, ketoconazole, diltiazem, amiodarone, aspirin and losartan.
The last creatinine level was 1.4 mg/dl, plasma bicarbonate concentration 23.2 mEq/L and proteinuria 255 mg/24 h.
